Criteria for the diagnosis of corticobasal degeneration

Melissa J. Armstrong, Irene Litvan, Anthony E. Lang, Thomas H. Bak, Kailash P. Bhatia, Barbara Borroni, Adam L. Boxer, Dennis W Dickson, Murray Grossman, Mark Hallett, Keith Anthony Josephs, Andrew Kertesz, Suzee E. Lee, Bruce L. Miller, Stephen G. Reich, David E. Riley, Eduardo Tolosa, Alexander I. Tröster, Marie Vidailhet, William J. Weiner

Research output: Contribution to journalArticle

542 Citations (Scopus)

Abstract

Current criteria for the clinical diagnosis of pathologically confirmed corticobasal degeneration (CBD) no longer reflect the expanding understanding of this disease and its clinicopathologic correlations. An international consortium of behavioral neurology, neuropsychology, and movement disorders specialists developed new criteria based on consensus and a systematic literature review. Clinical diagnoses (early or late) were identified for 267 nonoverlapping pathologically confirmed CBD cases from published reports and brain banks. Combined with consensus, 4 CBD phenotypes emerged: corticobasal syndrome (CBS), frontal behavioral-spatial syndrome (FBS), nonfluent/agrammatic variant of primary progressive aphasia (naPPA), and progressive supranuclear palsy syndrome (PSPS). Clinical features of CBD cases were extracted from descriptions of 209 brain bank and published patients, providing a comprehensive description of CBD and correcting common misconceptions. Clinical CBD phenotypes and features were combined to create 2 sets of criteria: more specific clinical research criteria for probable CBD and broader criteria for possible CBD that are more inclusive but have a higher chance to detect other tau-based pathologies. Probable CBD criteria require insidious onset and gradual progression for at least 1 year, age at onset ≥50 years, no similar family history or known tau mutations, and a clinical phenotype of probable CBS or either FBS or naPPA with at least 1 CBS feature.ThepossibleCBDcategoryuses similar criteria but has no restrictions on age or family history, allows tau mutations, permits less rigorous phenotype fulfillment, and includes a PSPS phenotype. Future validation and refinement of the proposed criteria are needed.

Original languageEnglish (US)
Pages (from-to)496-503
Number of pages8
JournalNeurology
Volume80
Issue number5
DOIs
StatePublished - Jan 29 2013

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Phenotype
Primary Progressive Aphasia
Progressive Supranuclear Palsy
Consensus
Neuropsychology
Mutation
Movement Disorders
Brain
Neurology
Degeneration
Age of Onset
Syndrome
Pathology
Research
Onset
Family History

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)

Cite this

Armstrong, M. J., Litvan, I., Lang, A. E., Bak, T. H., Bhatia, K. P., Borroni, B., ... Weiner, W. J. (2013). Criteria for the diagnosis of corticobasal degeneration. Neurology, 80(5), 496-503. https://doi.org/10.1212/WNL.0b013e31827f0fd1

Criteria for the diagnosis of corticobasal degeneration. / Armstrong, Melissa J.; Litvan, Irene; Lang, Anthony E.; Bak, Thomas H.; Bhatia, Kailash P.; Borroni, Barbara; Boxer, Adam L.; Dickson, Dennis W; Grossman, Murray; Hallett, Mark; Josephs, Keith Anthony; Kertesz, Andrew; Lee, Suzee E.; Miller, Bruce L.; Reich, Stephen G.; Riley, David E.; Tolosa, Eduardo; Tröster, Alexander I.; Vidailhet, Marie; Weiner, William J.

In: Neurology, Vol. 80, No. 5, 29.01.2013, p. 496-503.

Research output: Contribution to journalArticle

Armstrong, MJ, Litvan, I, Lang, AE, Bak, TH, Bhatia, KP, Borroni, B, Boxer, AL, Dickson, DW, Grossman, M, Hallett, M, Josephs, KA, Kertesz, A, Lee, SE, Miller, BL, Reich, SG, Riley, DE, Tolosa, E, Tröster, AI, Vidailhet, M & Weiner, WJ 2013, 'Criteria for the diagnosis of corticobasal degeneration', Neurology, vol. 80, no. 5, pp. 496-503. https://doi.org/10.1212/WNL.0b013e31827f0fd1
Armstrong MJ, Litvan I, Lang AE, Bak TH, Bhatia KP, Borroni B et al. Criteria for the diagnosis of corticobasal degeneration. Neurology. 2013 Jan 29;80(5):496-503. https://doi.org/10.1212/WNL.0b013e31827f0fd1
Armstrong, Melissa J. ; Litvan, Irene ; Lang, Anthony E. ; Bak, Thomas H. ; Bhatia, Kailash P. ; Borroni, Barbara ; Boxer, Adam L. ; Dickson, Dennis W ; Grossman, Murray ; Hallett, Mark ; Josephs, Keith Anthony ; Kertesz, Andrew ; Lee, Suzee E. ; Miller, Bruce L. ; Reich, Stephen G. ; Riley, David E. ; Tolosa, Eduardo ; Tröster, Alexander I. ; Vidailhet, Marie ; Weiner, William J. / Criteria for the diagnosis of corticobasal degeneration. In: Neurology. 2013 ; Vol. 80, No. 5. pp. 496-503.
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